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Use of subcutaneous treprostinil (TRE) in children with pulmonary hypertension (PH) secondary to lung disease (group 3 PH) is not well described. We hypothesized that initiation of TRE is associated with improvement in PH severity and right ventricular (RV) function with minimal early side effects. We conducted a single‐center retrospective analysis of children with group 3 PH treated with TRE between 2006 and 2022. We compared echocardiographic changes in PH severity and RV systolic function over the first 3‐6 months of TRE and described adverse effects over the first 48 h of therapy. Forty‐one patients (56% male), aged 6 days to 15 years old at the time of TRE initiation, were included. The most common associated diagnoses were bronchopulmonary dysplasia (63%), congenital diaphragmatic hernia (10%), and pulmonary hypoplasia (10%). TRE was associated with improvement in PH severity (p < 0.001), estimated by interventricular septal position at end‐systole (p = 0.001), RV systolic pressure to systemic blood pressure ratio (p < 0.001), and flow direction across a simple shunt (p = 0.153). There were improvements in individual markers of RV function including tricuspid annular plane systolic excursion (p = 0.005), tricuspid annulus dilation (p = 0.004), RV global longitudinal strain (p < 0.001), RV free wall longitudinal strain (p < 0.001), and qualitative RV systolic function (p = 0.002). Over the first 48 h of TRE, the dose was reduced in 3 patients (7%) due to hypotension, hypoxemia, or emesis, all of whom tolerated re‐escalation within 48 h. TRE is well tolerated and is associated with early improvement in PH severity and RV function in children with group 3 PH.

Children with severe Group 1 pulmonary arterial hypertension (PAH) have an unpredictable response to subcutaneous treprostinil (TRE) therapy, which may be influenced by age, disease severity, or other unknown variables at time of initiation. In this retrospective single‐center cohort study, we hypothesized that younger age at TRE initiation, early hemodynamic response (a decrease in pulmonary vascular resistance by ≥30% at follow‐up catheterization), and less severe baseline hemodynamics (Rp:Rs < 1.1) would each be associated with better clinical outcomes. In 40 pediatric patients with Group I PAH aged 17 days−18 years treated with subcutaneous TRE, younger age (cut‐off of 6‐years of age, AUC 0.824) at TRE initiation was associated with superior 5‐year freedom from adverse events (94% vs. 39%, p = 0.002), better WHO functional class (I or II: 88% vs. 39% p = 0.003), and better echocardiographic indices of right ventricular function at most recent follow‐up. Neither early hemodynamic response nor less severe baseline hemodynamics were associated with better outcomes. Patients who did not have a significant early hemodynamic response to TRE by first follow‐up catheterization were unlikely to show subsequent improvement in PVRi (1/8, 13%). These findings may help clinicians counsel families and guide clinical decision making regarding the timing of advanced therapies.

Purpose of Review Neuropathic pain is often debilitating, severely limiting the daily lives of patients who are affected. Typically, neuropathic pain is difficult to manage and, as a result, leads to progression into a chronic condition that is, in many instances, refractory to medical management. Recent Findings Gabapentinoids, belonging to the calcium channel blocking class of drugs, have shown good efficacy in the management of chronic pain and are thus commonly utilized as first-line therapy. Various sodium channel blocking drugs, belonging to the categories of anticonvulsants and local anesthetics, have demonstrated varying degrees of efficacy in the in the treatment of neurogenic pain. Summary Though there is limited medical literature as to efficacy of any one drug, individualized multimodal therapy can provide significant analgesia to patients with chronic neuropathic pain.

Transthoracic intracardiac lines provide a unique access point for postoperative monitoring and management in paediatric cardiothoracic surgeries, particularly within the single ventricle population where preserving vasculature is crucial for future interventions. This retrospective review examined paediatric single ventricle patients undergoing cardiothoracic surgeries at a tertiary children’s hospital between 2011 and 2018, focusing on the use of and factors associated with transthoracic line complications (infection, thrombosis, malfunction, and migration). A total of 338 lines were placed during the study period, with the majority occurring during palliative surgeries (86.5%). Lines remained in place for a median of 14 days postoperatively. Complications occurred in 21 lines (6.2%), comprising 8 migrations (2.4%), 7 thrombosis (2.1%), 4 malfunctions (1.2%), and 2 infections (0.6%). The presence of a surgical shunt was significantly associated with line complications (odds ratio 2.58, confidence interval 1.05 – 6.31; P 0.03). The use of transthoracic intracardiac lines seems to be safe and should be considered as a primary alternative to other central lines in the single ventricle population. A prospective assessment of transthoracic line complications, along with delineation of unit protocols, may further enhance outcomes in this complex population.

To characterize the use of right atrial lines (RALs) as primary access in the postoperative care of neonatal and pediatric patients after cardiothoracic surgery and to identify risk factors associated with RAL complications. Observational retrospective cohort study in pediatric cardiac patients who underwent RAL placement in a tertiary children’s hospital from January 2011 through June 2018. A total of 692 children with congenital heart disease underwent 815 RAL placements during the same or subsequent cardiothoracic surgeries during the study period. Median age and weight were 22 days (IQR 7–134) and 3.6 kg (IQR 3.1–5.3), respectively. Neonates accounted for 53.5% of patients and those with single-ventricle physiology were 35.4%. Palliation surgery (shunts, cavo-pulmonary connections, hybrid procedures, and pulmonary artery bandings) accounted for 38%. Survival to hospital discharge was 95.5%. Median RAL duration was 11 days (IQR 7–19) with a median RAL removal to hospital discharge time of 0 days (IQR 0–3). Thrombosis and migration were the most prevalent complications (1.7% each), followed by malfunction (1.4%) and infection (0.7%). Adverse events associated with complications were seen in 12 (1.4%) of these RAL placements: decrease in hemoglobin ( n  = 1), tamponade requiring pericardiocentesis ( n  = 3), pleural effusion requiring chest tube ( n  = 2), and need for antimicrobials ( n  = 6). Multivariable logistic regression showed that RAL duration (OR 1.01, p  = 0.006) and palliation surgery (OR 2.38, p  = 0.015) were significant and independent factors for complications. The use of RALs as primary access in postoperative pediatric cardiac patients seems to be feasible and safe. Our overall incidence of complications from prolonged use of RALs remained similar or lower to that reported with short-term use of these lines. While RAL duration and palliation surgeries seemed to be associated with complications, severity of illness could be a confounding factor. A prospective assessment of RAL complications may improve outcomes in this medically complex population.

Introduction: Hypercalcemia has a wide range of causes, and there are many important diagnostic values that must be considered by the pediatrician in reaching a diagnosis. Case and Discussion: This case provides an example for the outpatient-workup of hypercalcemia in pediatric population. The patient is a 17 m/o male with a three month episodic history of non-bloody, watery diarrhea and poor weight gain. He was born at 39 weeks gestation to a G2P2 mother. The pregnancy was complicated by DM-II and obesity. All newborn screening was normal. Initial testing revealed an elevated serum calcium level of 11.0mg/dL. In the initial workup of pediatric hypercalcemia, it is important to establish the presence of an appropriate physiologic response to elevated serum calcium. In patients with functional parathyroid glands, hypercalcemia should induce a low or suppressed level of parathyroid-hormone (PTH) secretion. N appropriate response suggests that the underlying cause of hypercalcemia is PTH-independent, such as hypervitaminosis D, sarcoidosis, or paraneoplastic secretion of PTHrp. If PTH levels are elevated, PTH-dependent causes of hypercalcemia must be considered, such as 1º/3º hyperparathyroidism and familial hypocalciuric hypercalcemia (FHH). Random urine calcium levels are useful in distinguishing between these causes, as calcium reabsorption in the kidney will hit a saturation point with 1º/3º hyperparathyroidism, resulting in detectable calciuria. Further testing in this patient revealed serum electrolytes, albumin, total protein, creatinine, and LFTs all within normal limits. PTH levels were elevated at 99.7 mg/dL, suggesting a PTH-dependent cause of hypercalcemia. Random urine calcium was collected and found to be significantly low at <1.0mg/24h, which suggests a diagnosis of FHH. The patient was referred to a radiologist for ultrasound of the neck to effectively rule out neoplastic-related 1º hyperparathyroidism. Conclusion: Evaluation of hypercalcemia is important and relevant to rule out parathyroid or other diseases. FHH is normally asymptomatic, and there are no curative treatments for this condition.